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Childhood extracranial germ cell tumors may be benign or malignant.

Malignant extracranial germ cell tumors are tumors that form outside the brain. They are gonadal or extragonadal.

Gonadal Germ Cell Tumors

Gonadal germ cell tumors form in the gonads (testicles and ovaries).

Testicular germ cell tumors. Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma. Nonseminomas are usually large and cause signs or symptoms of disease. They tend to grow and spread more quickly than seminomas.

Testicular germ cell tumors usually occur before the age of 4 years or in adolescents and young adults. Testicular germ cell tumors in adolescents (11 years and older) and young adults are different from those that form in early childhood.

There are three types of extracranial germ cell tumors.

Teratomas

There are two main types of teratomas:

Mature teratomas. These tumors are the most common type of extracranial germ cell tumor. Mature teratomas are benign tumors and not likely to become cancer. They usually occur in the sacrum or coccyx in newborns or in the testicles or ovaries at the start of puberty. The cells of mature teratomas look almost like normal cells under a microscope. Some mature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Immature teratomas. These tumors usually occur in areas other than the gonads in young children or in the ovaries at the start of puberty. They have cells that look very different from normal cells under a microscope. Immature teratomas may be cancer and spread to other parts of the body. They often have several different types of tissue in them, such as hair, muscle, and bone. Some immature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Malignant Germ Cell Tumors

Malignant germ cell tumors are cancer. There are two main types of malignant germ cell tumors:

Seminomatous germ cell tumors. There are three types of seminomatous germ cell tumors:

Seminomas form in the testicle.

Dysgerminomas form in the ovary.

Germinomas form in areas of the body that are not the ovary or testicle, such as the mediastinum.

Nonseminomatous germ cell tumors. There are five types of nonseminomatous germ cell tumors:

Yolk sac tumors make a hormone called alpha-fetoprotein (AFP). They can form in the ovary, testicle, or other areas of the body.

Choriocarcinomas make a hormone called beta-human chorionic gonadotropin (β-hCG). They can form in the ovary, testicle, or other areas of the body.

Embryonal carcinomas may make a hormone called β-hCG. They can form in the testicle or other areas of the body, but not in the ovary.

Gonadoblastomas.

Teratoma and yolk sac tumors.

Mixed Germ Cell Tumors

Mixed germ cell tumors are made up of at least two types of malignant germ cell tumor. They can form in the ovary, testicle, or other areas of the body.

The cause of most childhood extracranial germ cell tumors is unknown.

Having certain inherited disorders can increase the risk of extracranial germ cell tumors.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.

Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken.

Serum tumor marker test: A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.

Some malignant germ cell tumors release tumor markers. The following tumor markers may be used to detect extracranial germ cell tumors:

Alpha-fetoprotein (AFP).

Beta-human chorionic gonadotropin (β-hCG).

For testicular germ cell tumors, blood levels of the tumor markers help show if the tumor is a seminoma or nonseminoma.

Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.

Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.

Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Sometimes an incisional biopsy or needle biopsy is done before surgery to remove a sample of tissue. Sometimes the tumor is removed during surgery and then a sample of tissue is removed from the tumor.

The following tests may be done on the sample of tissue that is removed:

Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.

Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.

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